Sickle Cell Anemia

Sickle cell anemia is an inherited disease of the red blood cells. This disease has the highest prevalence in African Americans (approx. 8% of African Americans carry the gene trait). Patients who have this serious disease are known as sickle cell DOMINANT. Those who are carriers for sickle cell disease are known as sickle cell TRAIT. The patients with sickle cell trait do NOT have the problems associated with true sickle cell disease, but could pass the DOMINANT form of the disease to their offspring. Patients with sickle cell disease have an abnormal type of hemoglobin in their red blood cells. Hemoglobin is the molecule inside the red blood cell that allows it to carry oxygen. Sickle cell anemia is a "multisystem" problem, meaning that it can affect several different systems in the body. Blockage of small blood vessels occurs, resulting in many of the manifestations of sickle cell anemia. Below is a partial list.

MANIFESTATIONS OF SICKLE CELL ANEMIA

1. VISUAL CHANGES (blindness)

2. SEIZURES

3. CONGESTIVE HEART FAILURE

4. LUNG DISEASE

5. LEG ULCERS

6. PRIAPISM (prolonged, involuntary penile erection)

7. BONE AND JOINT PAIN (probably the most common complaint)

8. PLEURISY and BREATHING DIFFICULTY

Evaluation is by blood test which identifies the abnormal hemoglobin present in the cells of patients with this disease. The CBC shows anemia. There is NO treatment for sickle cell anemia. Only supportive care, to limit the symptoms, is available at this time. These patients also have an increased susceptibility for certain bacterial infections.

REMEMBER

People who are sickle cell TRAIT have for the most part no serious health problems and will not have the same problems as those who are sickle cell DOMINANT. Sickle cell DOMINANT patients have a much poorer prognosis, and survival into adulthood is more unusual.

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